Anesthetic considerations in primary immunodeficiencies

Primary immunodeficiencies are a group of mostly hereditary, or congenital, disorders. Some cases, however. show no hereditary pattern despite an evident familial distribution. The incidence of these cases is variable and the most frequent of them is immunoglobulin A deficiency. Many are pediatric disorders that are occasionally so serious that the patient does not survive the first year of life due to the development of systemic infections. In other cases, survival is much longer and it is possible to find adult carriers in routine clinical practice. These are less aggressive cases that form part of specific clinical syndromes that must be recognized so that appropriate anesthetic management can be planned. We review the clinical characteristics of primary immunodeficiencies that may be relevant to anesthetic management in these patients.